Huperzine A, Mestinon and Myasthenia Gravis

Huperzine A gets a lot of attention as a natural (and sometimes necessary) alternative to pyridostigmine bromide, especially when there are adverse side effects to the synthetic treatment option. So what is the best choice and how do we begin to navigate the options in front of us?

First of all, let’s define what exactly Huperzine A is. Huperzine A is a dietary supplement that is “derived from the Chinese club moss Huperzia serrata and works as a cholinesterase inhibitor — a type of medication that works by improving the availability of the neurotransmitter acetylcholine”, needed for muscle contraction. (1) By definition, this is a similar mechanism of action found in pyridostigmine, also known by the brand name of Mestinon. Pyridostigmine is also classified as a cholinesterase inhibitor which helps to inhbit “the destruction of acetylcholine by cholinesterase and thereby permits freer transmission of nerve impulses across the neuromuscular junction”. (2)

While Huperzine A has been used for centuries in the Chinese culture to treat everything from “rheumatism and colds, to relax muscles and tendons, and to improve blood circulation”, it gained attention as a potential use in the treatment of Alzheimer’s patients. (3) In recent years however, it has sparked interest in the potential treatment of Myasthenia Gravis as an alternative to pyridostigmine. Many cite adverse side effects and intolerable reactions to the pyridostigmine as a motivator for switching to Huperzine A. Others still prefer a more natural approach to their medication regimen.

        

It is important to know that Huperzine A has only been clinically studied, and at specific doses, in relation to Alzheimer’s disease. This has no clinical backing in the potential treatment of Myasthenia Gravis in the way of controlled studies. (3)

Let’s dig a little deeper though into how these two treatments break down in our bodies and are used to either our benefit or detriment.

  • Side effects, adverse reactions and cholinergic crisis:
    Within the Myasthenic communities online, you may stumble upon individuals who have made the switch from the synthetic pyridostigmine to the natural Huperzine A, citing they could not tolerate an ambiguously defined set of side effects and/or adverse reactions. This is very important to talk about and unpack as much of the movement in favor for Huperzine A rests right here. Pyridostigmine bromide works by stimulating the parasympathetic nervous system, which inevitably includes an over stimulation of the gastrointestinal tract that can lead to bloating, cramping and diarrhea. (4) For many, these side effects can be harsh and debilitating both inside and outside the home and leave many with social hesitation or anxiety of being too far from the nearest bathroom. Pyridostigmine can also cause some mild muscular twitching and systemic cramping, although these side effects are notably and significantly less than their GI counterparts. (4) Understandably so, this would motivate anyone who experiences such side effects to seek a potentially more gentle alternative that is billed as safer and effective for those who made the switch.

 

  • But, we need to talk about definitions here. Few have ever clarified if they experienced the above reactions and subsequently classified them as “adverse” or “side effects” or if there is more to the picture than meets the eye. You see, pyridostigmine, when taken in a greater dose than what is needed*, can cause what is called a cholinergic crisis which has the potential to produce adverse effects and potentially life threatening scenarios in it’s most severe form. These effects can come about in rapid sequence (usually they begin 45-60 minutes after ingesting an oral dose) and not all symptoms may present in all individuals. Moderate to severe twitching, miosis, excessive salivation and lacrimation, nausea, vomiting and severe diarrhea, severe GI cramping, difficulty breathing, swallowing and speaking, sweating and flushing are all part of the potential presentation. (4) Since it can mimic in presentation with a Myasthenic crisis (disease induced rather than medication induced), it is not always easy to pick up on, even with a trained eye, and can leave many assuming that it is simply an unpleasant side effect of the drug itself. This lack of clarity between true side effect and cholinergic crisis, even in a more mild to moderate form, leaves the Myasthenic with some of the puzzle pieces missing. *Please note that overdose is not the fault of the patient in that it is rarely intentional. As environmental factors and immune fluctuations occur, the need for pyridostigmine can vary and the lack of adjustment to those influencing factors often are the culprit in producing cholinergic crisis.
  • Huperzine A, as noted above, has the same mechanism of action as does it’s synthetic counterpart, meaning that you have the exact same potential to repeat the side effects and cholinergic crisis risks as you did with pyridostigmine. It may take a much higher dose and the impact may be slightly minimized, but the risks are all still there. The selling point it is safer since it is natural. Again, we need to go back to definitions in order to get a clearer picture of what safer with any treatment really means. As it currently stands, “safer” is a loaded word with ambiguous meaning here. We have already talked about it being in the same class as pyridostigmine and working on the same mechanism which, ergo, offers the same risks. Huperzine A still must be processed by the kidneys and liver, just like the synthetic version and it’s renal toxicity is almost identical. (3,4) But we must also recognize that, as a dietary supplement, it is unregulated and that makes it more difficult to ensure safety, efficacy and viability. There are no studies or trials that offer patients or treating physicians a standard for dosing, peaks and falls like we see in pyridostigmine, further augmenting the potential for cholinergic danger and under dosing.
  • The typical half life of pyridostigmine bromide is 3- 4 hours after renal elimination with an estimated half life of Huperzine A to be an estimated 10-14 hours after renal elimination. (3,5) This astounding difference in half life clearance solidifies a marked difference in safe dosing regimens, further augmenting the concerns over the lack of regulation in ingredients and the absence clinical studies to offer up a clear understanding of integral information (ie., when peak release and half-life begins and ends etc..). This leaves both patient and physician zero dosing standard and narrowly contributes a vague and unchecked approach to treatment.

Lack of access, cost and delayed diagnosis can leave many without alternative options other than to reach for Huperzine A and there are those who firmly believe that Huperzine A helps them control their Myasthenia Gravis with fewer side effects than pyridostigmine. While these scenarios and individuals may find great success with their switch to Huperzine A, it begs discernment and caution in understanding all of the potential pros and cons before moving forward.

Author: Rebekah Dorr
Founder & Administrator, Myasthenia Gravis Unmasked

References:
(1) (http://www.mayoclinic.org/diseases-conditions/alzheimers-disease/expert-answers/huperzine-a/faq-20058259)

(2) (Miller RD. Pharmacodynamics and pharmacokinetics of anticholinesterase. In: Rueg-heimer E, Zindler M, ed. Anaesthesiology. [Hamburg, Germany: Congress; Sep 14-21,1980; 222-223.] [Int Congr. No. 538], Amsterdam, Netherlands: Excerpta Medica; 1981)

(3) (https://www.drugs.com/pro/pyridostigmine.html)

(4) (http://www.mims.com/malaysia/drug/info/pyridostigmine%20bromide/?type=brief&mtype=generic)

(5) (https://www.medicines.org.uk/emc/medicine/865)

Help Tyler Get a New Mobility Scooter

Hi, I’m Tyler, the admin of the MG Hope blog. I have MG among many other chronic illnesses and I use a mobility scooter to get around on my college campus.

I’m a part time college student studying for a B.S. in Computer Information Systems & Technology. I started college in 2011 as a full time student before my diagnosis and kept going while trying to get a diagnosis. I am determined to finish and my mobility scooter is my lifeline to get to and from classes and meetings as a student leader. Without a new scooter, I will likely be unable to finish school.

My current scooter needs about $500 – $600 worth of parts and it is 15 years old, bought used. I need a new one as I can’t see putting money into something that may or may not last, but I lack the funds to buy it. My insurance won’t pay for it as I don’t need or use it within my home to complete my “daily living activities.”

I greatly appreciate any help you can provide and I hope you’ll consider sharing this link.

https://www.youcaring.com/tylermorris-801596

Thanks,
Tyler
MG Hope Admin

In order to combat spam…

You will now see what’s called a Captcha (in this case images containing numbers that you must add, subtract, or multiply) in the comments form, the login form, and most other forms on the site.

We want to keep this blog clean and a good resource of information about MG by removing/blocking as much spam as possible. I appreciate your understanding.

Myasthenia Gravis Showed Me a New Purpose

“Ma, I can’t open the car door.” Those words were my introduction to Myasthenia Gravis. As my mom looked at me she saw my eyelids began to droop right before her eyes. Trying to open the car door again, my hands slipped. There was no grip at all. At that moment I was rushed to the hospital.

The tensilon test was how I was diagnosed. Upon the injection my eyes flew open and my hands were strong. I don’t remember anything else that day because I was scared. I was 12 years old and documented as the youngest MG patient at Stony Brook University Hospital in NY. It was 1989.

Fast forward two years. I had a thymectomy. I was 14. Most of high school I was home schooled with so many doctors appointments and being sick a lot.

Time went on but my fears were dwindling as I became more educated about MG. I almost died twice due to complications from MG, but I am still here! Then I realized I had a purpose, to help support others with the same illness.

Now at 41, my symptoms have progressed to difficulty walking, climbing, swallowing, toileting, speaking, basically every human function. However, with God’s love, my husband, friends, and my mg support group, I still push on.

I can’t stay home alone anymore so I go to an adult center, but I’m using my story to help others at the center know they can push through. I don’t know what will happen, but I want my fight to be my legacy.

-Shirrel Miles

shirrel and rebekah

Shirrel and Rebekah at an MG Support Group Meeting

 

One Doctor Took a Chance and Saved My Life

Like many, my diagnosis was not easy to come by. I had traveled down the road of uncertainty, fear and anger before finally being diagnosed with yet “another disease”. I often wondered if this was indeed the one I had, the one that was making my body a stranger that I met in the dark alley that beat me up and changed me forever. I had been misdiagnosed more times than I could count. I rode the crazy train too, being the number one passenger. Especially if you asked numerous doctors who had given me the one way pass with no return. But I kept pushing, fighting, forcing doctors to listen to my anguish and tears.

I almost gave up, until one day I went to see my retina specialist. This dear doctor had seen me through many stages of my undiagnosed illness. The good, the bad and the ugly, he had met all forms of it. He kept saying there had to be more that was being missed. I will never forget the words he spoke to me the day that changed my life. He said “I’m going to give you some medicine. Will you be willing to take it and see if you notice any difference? It’s off the chart, just between us. I’m not giving you a name, I just want you to fill it and try it and call me in the morning.”

I thought sure, what the heck. I don’t even care. Maybe it will kill me. I’m so tired of fighting to find a diagnose that I don’t care if it works or knocks me out.

I had it filled and had my mom watch me. It was an instant change. It was as if I were superwoman! I came from sitting in a wheelchair to being able to move and talk and feed myself all within the same night. What was happening? Was this a fluke? What in the world did he give me? I wasn’t sure what to think. I had my doubts and questions but all I knew is that it felt as if he just saved my life, and I was thankful.

I called him the following morning and was so excited I couldn’t stand it! I relayed the great news. I’m not sure who was more excited, me or my doctor who had tried so hard to help me when no one else would. He had been seeing me twice a month for the longest time. He was my sounding board and sanity who tried to help me figure it all out. He said ” Felicia, you have Myasthenia Gravis. It was a long shot that we tried this and I believe, my dear, we finally have an answer for you. I need you to see a neurologist and have him confirm this diagnosis”.

What?! You want me to see another neurologist? I had seen everyone in the county and they had all written me off and said it was in my head. After all, being in your early 20’s apparently gave me zero credibility in the way that I felt. I was scared to see yet another neurologist, but I did. It was the best thing that I could have ever done. I saw Dr. Weiss with the MDA and he confirmed the diagnosis and was astounded that everyone else had missed this. EVERYONE. I started on mestinon and was scheduled for a thymectomy (major surgical procedure to remove an organ called the thymus gland that is believed to play a role in MG) all in a matter of months. I had a complete transternal thymectomy and was terrified. Scared again of something going wrong and what if they had been wrong and they slice and dice me for nothing? But in January of 2009, I underwent my surgery and came out stronger than I had ever been in my life. After my recovery of course! I went into remission. My life changed forever that year. I had my life back. I could be the mother and wife I had always wanted to be.

Sadly, it all came to a crashing halt when my “honeymoon period” was over. Remission lasted a year and a half for me. I was back full blown with my worst enemy. It’s been a bumpy road since then but I have learned more and I’ve become the person who knows what a real, life-long struggle is. I know that not everyday should be taken for granted. I know that the struggles are real and valid and that I can overcome each day by starting again the next. It may be my reality that I have to sleep for a week straight. Maybe I can’t cook, or my husband has to feed me my meals. Maybe I can’t walk the dog. Maybe I Netflix all day and pretend I’ve had an exciting day when in reality it’s just another day of struggling.

I do. I survive. I fight. I have Myasthenia Gravis. I’m proud to say that. I’m proud I have a name to a disease that almost took my life before I even knew what it was. I’m glad to have a name that ends the battle of all battles- the unknown. Now I just battle one thing. I have Myasthenia Gravis, I will survive and fight until my last breath.

Felicia Smith

Age 36

Wife, Mother, Daughter & Fighter!

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Story edited by Rebekah Dorr

I Thought Myasthenia Gravis Was No Big Deal

2012 – While driving one day, the white lines on the road seemed to multiply and I wasn’t sure of the lane I was driving in. Shook it off and went home. The next day I was playing golf and while looking down at the ball noticed that there were 2 balls… not a ball and a shadow but 2 balls. I had to reach down to determine which one was real.

Since I was experiencing vision issues I went to an ophthalmologist. She sent me to a neurologist. Neither doctor knew what was wrong. After multiple visits the ophthalmologist sent me for a blood test which provided her with the information to properly diagnose me with ocular Myasthenia Gravis. She said I should take the test results back to my neurologist – which I did.

The neurologist prescribed 40mg of prednisone and 60 mg of mestinon 4 times a day. My symptoms went away. The doctor weaned me off the prednisone and after a while the double vision returned. He increased my mestinon to 120mg 4 times a day and my double vision ceased.

I was on the medication for a few years, no more double vision, and continued all my activities as before. I did not think about MG or was concerned about it. Subsequent follow ups with the neurologist consisted of checking for arm and leg weakness…there was none. I was fine.

But in October 2015, I started to have some swallowing issues. Nothing too dramatic, just sometimes I had a problem swallowing a piece of food and I would regurgitate it to clear my throat. Planning on going to see family in Colorado for the holidays I self prescribed some prednisone for myself.
No big deal, right?

I developed a cold but still flew to Colorado. The temperature when I boarded the flight in Florida was 87 degrees; when I landed in Denver it was a mere 6 degrees.

Over the next 24 hours my swallowing became more difficult, my cold got worse and eventually I had my daughter take me to the ER. While answering questions checking into the ER my voice started to slur to the point that I was not intelligible. I was admitted into the ICU with pneumonia, and because I told the ER doctor I had ocular MG, he had a neurologist see me. By then I could swallow nothing, speech was labored and my oxygen level dropped.

Although they attempted to give me medication with apple sauce, pudding, etc…. I could not get it down. They eventually put a tube down my nose into my stomach to provide me with medication and nourishment. I fought off being intubated and kept my oxygen level up to an acceptable level. I then had IVIG every day for 5 days, they gave me 120 MG of prednisone (and told me to go off it when I left the hospital), 1000mg Cellcept 2x a day and gave me antibiotics to fight the pneumonia.

I remained in ICU for 7 days and a regular room in the hospital for another day. MG was no longer no big deal. I rested in Colorado for another week before flying back to Florida.

Since then, I am now on 20 mg prednisone and still on the 120 mg Mestinon 4x a day as well as the Cellcept, I see an MG specialist and am plugged in to a local support group.

I feel I have a new normal now. I play 9 holes of golf twice a week, limit my other outdoor activities and rest when I can. I eat softer foods, and much slower. I have not had a steak since 2015, as my chewing gets difficult after a while. I also eat earlier than I used to. (Nothing but soft foods, i.e. pudding, yogurt, etc. after 7pm.) When I look at a menu, I do not look at the description of the food as much as thinking about how chewable it is and whether or not I could swallow it.

There are some days when I just feel weak. Since I am retired – and no commitments to speak of, I am able to take those days as my body needs me to and just take it easy. I limit my evening activities and sometimes will be out with friends and just participate by listening. Whereas I was once the center of attention, dominating conversations I now limit my talking in the evening. I still sometimes have slurred speech but nothing I can’t deal with.

I have MG and am very conscious of it.
I think before I act and live with a “new normal”.
MG is no longer just no big deal.

– Stan Kotzker, Myasthenia Gravis Warrior

Story edited by: Rebekah Dorr

“I Am Not The Same Person As Before MG”: An Interview with Tyler Morris

I Am Not The Same Person As Before MG

MG Unmasked: What is Myasthenia Gravis to you?

Tyler: Myasthenia gravis is a neuromuscular/autoimmune disease that affects the way I walk, see, smile, and breathe. It is a disease that completely changed my life in both good and bad ways. It not only affected my physical being, but my emotional and mental being.

MG Unmasked: How has it changed your life both positively and negatively?

Tyler: MG has changed my life in many ways, both positively and negatively. On the negative side I struggle with daily tasks, I’ve lost friends, and I can no longer do things spontaneously. I struggle with walking quite a bit and use a cane. Most of the friends I had before I became ill slowly faded out of my life. They just don’t understand MG and what it’s like to live with a chronic illness. They expect me to be able to do everything I used to do and I just can’t. That has been difficult for me at times. I would love to be called the day of and go do something with friends or just hangout, but I can’t really do that anymore. Everything must be planned out, is it an accessible place, are there stairs/elevators, will I have to walk far, will I have to stand or sit for too long, can I drive there (how far is it) and is there handicapped parking?

On the positive side, I have met so many wonderful people that have been extremely supportive and helpful with my questions. I have gained access to what sometimes feels like an exclusive club, the MG community. Another positive thing is that I’ve slowed down (against my will) both literally and figuratively, and have learned to appreciate the little things in life. I’m so grateful to have met such wonderful people that not only understand MG, but understand me too, like my online friends, but also some friends from college. They’re the best. Do I wish I wasn’t sick? Of course, but would I change my experience? No.

MG Unmasked: MG hasn’t kept you from pursuing a college education. How do you make that work for you?

Tyler: Before my diagnosis, I had started my undergraduate degree, a BS in Computer Information Systems and Technology. I was a very active member of my campus community, participating in various activities, being president of our LGBTS Alliance, serving as an orientation counselor to incoming students for a few years, working a work study job, and taking 12 credits (full time) per semester.

While trying to get diagnosed, I was stuck using a powered scooter to get around our campus, even though it is tiny compared to most. My grades slipped a little and I had to withdraw from most of my courses, but I stayed in school throughout it all. I was determined to finish what I had started, taking just 3 credits a semester in an effort to get the 120 credits needed to graduate. I was also still in my work study job, however I soon had to quit as I couldn’t make it to work much and do one class a semester.

After my diagnosis, I’m still in college part time taking 3 to 6 credits a semester. I do receive accommodations and my professors are usually very understanding of my illness. I ask for leniency with attendance and occasionally extensions to get work done. I live on campus with an amazing roommate. He’s very understanding and accepting of my illness and me in general. He does a lot for me, including cleaning the dorm (most of the hard work), and taking out the trash and recycling as I can’t carry it long enough to get to the dumpster/recycling bins. While I have slowed down quite a bit, I’m still president of the LGBTS Alliance which is often a lot of work, but my fellow officers are very helpful and often take over when I can’t be at an event or meeting. I’m also part of a diversity committee to the president of our campus and have been since it was formed in 2013. While I occasionally need my powered scooter still, many days I get around with just my cane. Overall college is a lot of work, but I’m determined to finish what I started and that determination is what drives me to keep going.

MG Unmasked: What advice would you offer someone else with MG who is trying to achieve their dreams in spite of it all?

Tyler: My advice would be to take it slow, know your limitations, and ask for help whenever you need it. I understand that may take some time, but I personally believe it is necessary in order to keep going.

MG Unmasked: What do you wish others understood most about MG and your life now?

Tyler: I wish others understood that I am not the same person I was before my illness. Any chronic illness can change your entire being. I wish they understood just how difficult it is to be exhausted all the time, to be weak most days, and still have to function. I wish they knew the fatigue that comes with myasthenia gravis and the fact that it is a chronic illness that is not going to go away.

Written by Rebekah Dorr of Myasthenia Gravis Unmasked.
Interview may be shared with credit to original author.

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Tyler Morris, Myasthenia Gravis Warrior